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SP0207 Scleroderma-Like Sclerosing Diseases
  1. A. Tyndall
  1. Rheumatology, University Hospital Basel, Basel, Switzerland

Abstract

Juvenile localised scleroderma and juvenile SSc have both have recently undergone classification revision. Essential to their diagnosis is the exclusion of other sclerosing disorders, most of which are rare.

Eosinophilic fasciitis (Schulman's disease) is usually symmetrical and tends to spare the face and hands, The absence of Raynauds, vascular changes and ANA distinguish it from scleroderma. Deep biopsy to and including muscle are required and peripheral eosinophilia is not always present. Also sparing the face, but variable in distribution is nephrogenic systemic fibrosis due to gadolinium in renal impaired patients including children. Scleredema of Buschke is mostly symmetrical, often involves the back (unlike scleroderma) and in children may follow respiratory infections. It often resolves spontaneously. It should be distinguished histologically from the neonatal panniculitides; sclerodema neonatorum (often fatal) and subcutaneous fat necrosis of the newborn. Sclerodema-like syndromes also may be seen in poorly controlled IDDM. Other metabolic/endocrine disorders with skin thickening include thyroid dysfunction and porphyria cutanea tarda.

Stiff skin syndrome starts in childhood with insidious onset of stony-hard skin, often with associated contractures, joint restriction and hypertrichosis. Biopsy shows fascial sclerosis and/or subcutaneous septa with increased fibroblast cellularity with sclerotic collagen bundles in the deep reticular dermis and no inflammation.

Scleroderma-like skin changes in infants, associated with growth retardation and dysmorphic features, suggest premature aging syndromes (Werners, Hutchinson Gilford, restrictive dermopathy), requiring genetic testing and counselling of asymptomatic carriers of LMNA mutations.

Infections (e.g.borrelia burgdorferi) and exposure to toxins should be excluded in all skin thickening syndromes.

Disclosure of Interest None declared

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